Abstract
Congenital midline cervical cleft is a rare anomaly and is clinically apparent at birth. Histology of this defect is consistent with the presence of stratified squamous epithelium. However, we present a case of 1-year-old boy with chronic mucocutaneous candidiasis associated with two cysts and presence of focal respiratory epithelium. We attempt to discuss the presentation, the histological differences, and the optimal surgical treatment for the same.